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R3 case conference

R3 case conference. 報告者 : 楊仁星 指導老師 : 方基存醫師 報告日期 :2011-11-2. Index. Case presentation Discussion Back to the case. Case Presentation. Patient ’ s Profiles. Name: 陳 x 羽 Gender: female Age: 58 years Ethnic: Taiwanese Marriage: married Occupation: Waitress, School janitor, 有線電視服務員

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R3 case conference

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  1. R3 case conference 報告者:楊仁星 指導老師:方基存醫師 報告日期:2011-11-2

  2. Index • Case presentation • Discussion • Back to the case

  3. Case Presentation

  4. Patient’s Profiles • Name: 陳x羽 • Gender: female • Age: 58 years • Ethnic: Taiwanese • Marriage: married • Occupation: Waitress, School janitor, 有線電視服務員 • Chart number: 39037xxx • Date of admission: 2011-9-26

  5. Chief complaint • General weakness for 4 days

  6. Present Illness-I • The patient had weakness of bilateral lower limbs for several days • 4 days before admission, the weakness was progressed to general weakness. • She received injection of unknown substance (may be analgestic) at LMD.

  7. Present Illness-II • After injection, the weakness became worsen and then the patient couldn’t walk and stand. • The associated symptoms included palpitation. • She came to our ER for help

  8. Present Illness-III • She denied fever, chest pain, chest tightness, cold sweating, dyspnea, dizzness, nausea, vomiting, diarrhea, anorexia, tarry or bloody stool, frequency, urgency and flank pain.

  9. Past History: • Diabetes mellitus for half year without control • Denied hypertension, heart disease, HBV, HCV and operation history

  10. Personal history: • Allergy: no known allergy • Alcohol: denied • Smoking: denid • Betelnut: denied • Medication history: Chinese Medicine (大豆類黃酮,保護關節藥)

  11. Family History:

  12. Physical Examination-I • Vital sign: BT: 36℃, PR:62/min, RR: 18/min, BP: 121/80 mmHg • General appearance: fair looking • Consciousness: alert, E4V5M6 • HEENT: sclera: not icteric, conjunctiva: not pale • Neck: supple, JVE (-/-), LAP (-/-) • Chest: bilateral symmetric expansion bilateral breathing sound: clear • Heart: regular heart beat without murmur

  13. Physical Examination-II • Abdomen: flat no superficial vein engorgement no spider angioma normoactive bowel sound no tenderness, no rebound pain no Murphy’s sign liver and spleen not palpable no shifting dullness • Back: no knocking pain over bilateral flank pain • Extremities: no pitting edema at bilateral lower limbs • Skin: no petechiae or ecchymoses no skin rash

  14. EKG at ER

  15. CXR at ER

  16. Lab data-I

  17. Lab data-II

  18. Impression • Hypokalemia periodic paralysis • Diabetes mellitus

  19. Clinical course-I

  20. Clinical course-II • Other blood tests during hospitalization

  21. Clinical course-III • Urine anion gap: 56+14.1-62 = 7.9

  22. Clinical course-III kidney echo on 9/30

  23. Clinical course-IV kidney echo on 9/30

  24. Clinical course-Vkidney echo on 9/30 • Left Kidney Length: 10.7 cm • Right Kidney Length: 11.8 cm • Impression: • Right renal stone (0.7cm) • Bilateral renal calcification spots • Parenchymal renal disease

  25. Clinical course-IV

  26. Final diagnosis • Type I renal tubular acidosis, suspect autoimmune disease related • Renal stones • Type II diabetes mellitus, under diet control

  27. Discussion

  28. Renal tubular acidosis

  29. Renal tubular acidosis • A systemic hyperchloremic and normal anion gap acidosis with relatively normal glomerular filtration rate • Results from either the net retention of hydrogen chloride or net loss of sodium bicarbonate • Three major subgroups of RTA • Distal or type 1 RTA • Proximal or type 2 RTA • Hypoaldosteronism or type 4 RTA

  30. Renal tubular acidosis-distal RTA (type 1) • Failure of distal nephron and collecting duct to secret hydrogen ion • Failure to reabsorb filtered bicarbonate that was not reabsorbed in proximal tubule • Failure of titration of phosphate buffer and decreased exc • retion of NH4+ • Precise nature of defect is not known.

  31. dRTA (Type I)

  32. dRTA (Type I)- Etiology

  33. dRTA (Type 1)- Clinical features • muscle weakness • Hyperventilation • Acute acidosis • Hypokalemia • Inability to acidify the urine to a pH of less than 5.3 • 70% have either nephrocalcinosis (very rarely a feature of other types) or calcium containing renal calculi • Rickets and growth stunting are frequent features in childhood cases. ± osteomalacia in adults

  34. Renal tubular acidosis-proximal RTA (type 2) • Proximal tubule reabsorption of approximaly 80~90% • Type 2 RTA: • an impariment of HCO3- reabsorption in the proximal tubule • Decreased renal HCO3- threshold • Distal acidifcation mechanisms are intact • May lower urine pH below 5.5

  35. pRTA ( Type 2)

  36. pRTA ( Type 2): Etiology

  37. pRTA ( Type 2): Clinical feature • General weakness • Metabolic acidosis • Hypokalemia • proximal myopathy, osteomalacia or rickets • Normal urinary acidification • Nephrocalcinosis and renal calculi are virtually never present

  38. Renal tubular acidosis-type 4 • Type 4 RTA is not actually a tubular disorder • reduction in proximal tubular ammonium excretion, which is secondary to hypoaldosteronism or pseudohypoaldosteronism, and results in a decrease in urine buffering capacity. • Presentation: hyperkalemia and normal urinary acidification • Nephrocalcinosis and Urolithiasis are absent in type 4

  39. Hyperkalemic RTA (Type 4): Etiology

  40. Renal tubular acidosis

  41. Sjogren’s syndrome

  42. Sjögren's syndrome • A chronic autoimmune disease, affecting mainly the exocrine glands. • Prevalence: 0.3%~0.6% • Female:male: 9:1 • Can affect extraglandular, such as involvement of the musculoskeletal, pulmonary, GI, hepatobiliary, hematologic, vascular, dermatologic, renal and nervous systems.

  43. Sjögren's syndrome-Classification • Primary SS: occur alone • Secondary SS: association with another defined autoimmune disease (eg, SLE, RA, or scleroderma)

  44. Sjögren's syndrome-Symptoms • Two main symptoms: dry eye and dry mouth • Other: • Joint pain, swelling and stiffness • Swollen salivary gland • Skin rashes or dry skin • Vaginal dryness • Persistent dry cough • Prolonged fatigue

  45. Diagnosis Criteria • 2002 American-European consensus Classification Criteria, required four of the following

  46. Renal involvement in primary Sjogren’s syndrome

  47. Sjogren’s syndrome is an autoimmune disorder and the target organs are the exocrine glands, especialy the lacrimal and salivary • Kidney involvement is a frequent extraglandular manifestation of primary Sjogren’s syndrome • 30~40% of Sjogren’s syndrome patients have symptomatic and asymptomatic renal involvment. • The understanding of the clinical presentation of renal involvement in primary Sjogren’s syndrome is based on case reports and small retrospective cohorts.

  48. Clinical and laboratory features

  49. Clinical and laboratory features • Patient with Sjogren’s syndrome and RTA tend to present hypokalemic periodic paralysis (HPP)

  50. 20 cases of SS-associated HPP reported between 1966~2008

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